517936
research-article2014
IJSXXX10.1177/1066896913517936International Journal of Surgical PathologyHatanaka et al
Case Report
The Hyaline Vascular Type of Castleman’s Disease of the Ovary: A Case Report
International Journal of Surgical Pathology 2014, Vol. 22(7) 652–655 © The Author(s) 2014 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/1066896913517936 ijs.sagepub.com
Kazuhito Hatanaka, MD1, Takako Yoshioka, MD1, Takashi Tasaki, MD1, and Akihide Tanimoto, MD1
Abstract Castleman’s disease in the pelvic cavity is rare. We present a 72-year-old woman with hyaline vascular type of Castleman’s disease of the right ovary. Right ovarian enlargement was detected in the medical examination. Computed tomography revealed a solid mass, measured 2.5 cm in size, in the right ovary. A bilateral salpingo-oophorectomy was performed. Morphologically, lymphoid follicles with regressed germinal centers (GCs) were surrounded by a broad mantle zone composed of concentric rings of small lymphocytes, and the hyalinized blood vessels with plump endothelial cells penetrated radially into GCs. Proliferation of follicular dendritic cells, which were positive for CD21 and epidermal growth factor receptor, were detected in GCs and mantle zone. No other lesions were found. To the best of our knowledge, this is the first case of hyaline vascular type of Castleman’s disease of ovarian primary. Keywords Castleman’s disease, hyaline vascular type, ovary
Introduction Castleman’s disease (CD) is histologically divided into 2 forms: hyaline vascular (HV) type, which is the predominant form that accounts for approximately 90% of cases, and plasma cell type.1 The HVCD frequently occurs in a thoracic lymph node, although other rare lesions (both nodal and extranodal) also develop in the larynx, vulva, intrapericardial, intracranial, subcutaneous, and intramuscular location, and so on.2 We present the first case of HVCD that primarily occurred in the ovary.
Case Report A right ovarian enlargement was detected in a 72-year-old woman in the health examination. Computed tomography revealed a solid mass of 2.5 cm in size located in the right ovary. No other masses, lymphadenopathy, and surrounding invasion were noted. A bilateral salpingo-oophorectomy was performed under laparoscope. Macroscopically, the cut surface of the resected right ovary showed whitish-yellow and solid tumor with a distinct border, approximately 2.5 cm in diameter (Figure 1). No hemorrhage and necrosis were identified. There were no findings of invasion to the adjacent organs and structures including paraovarian lymph nodes. Besides the right fallopian tube, the left adnexa were intact.
Figure 1. Macroscopic view of the ovarian mass. The cut surface of the right ovary was whitish-yellow and solid with a distinct border.
Microscopic examination showed that round or irregular-shaped lymphoid follicles proliferated with small 1
Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan Corresponding Author: Akihide Tanimoto, Department of Molecular and Cellular Pathology, Kagoshima University Graduate School of Medical and Dental Sciences, 8-35-1 Sakuragaoka, Kagoshima 890-8544, Japan. Email: [email protected]
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Figure 2. Microscopic view of the ovarian mass. (A) Round or irregular-shaped lymphoid follicles with regressed germinal centers are proliferated in the ovarian tissue (arrow) (hematoxylin and eosin [H&E] stain, 4×). (B) Hyalinized blood vessels with plump endothelial cells radially penetrate into the follicles with concentric layer of mantle zone lymphocytes (“onion skin”) (H&E stain, 40×). (C) The germinal center is partially depleted of small lymphocyte and composed of follicular dendritic cells (H&E stain, 60×).
sized germinal centers (GCs), and normal ovarian tissue, including corpus albicans, was located in periphery (Figure 2A). There are some follicles with no GCs or more than one GC. Hyalinized blood vessels with plump endothelial cells penetrated radially into the follicles and a broad mantle zone, composed of concentric rings of small lymphocytes, was observed (Figure 2B). The GCs are depleted of small lymphocytes and composed of follicular dendritic cells (FDCs; Figure 2C). Hyaline deposits were also found. In the interfollicular area, small lymphoid cell infiltration, plasmacytoid dendritc cells clusters, and blood vessels proliferation lined by plump endothelial cells, but no prominent plasma cell infiltration, were identified. There was no rim of normal lymph node around the lesion. Immunohistochemically, the follicles were composed of CD20-positive B-cells. CD21 immunostaining revealed expanded FDCs network (Figure 3A).
Moreover, epidermal growth factor receptor (EGFR) expressed in FDCs (Figure 3B). The lymphocytes in the GCs were negative for Bcl-2 and cyclin D1. A few scattered plasma cells were polyclonal; immunoglobulin heavy chains (IgG, IgA, and IgM), and immunoglobulin light chains (κ and λ) were present with approximately equal distribution. Furthermore, plasma cells were negative for IgG4. T-cells positive for CD3 were predominantly found around the follicles.
Discussion Only few cases of CD in the female pelvis have been reported.3-5 They occur in the retroperitoneal space adjacent to the lateral pelvic wall as well as in presacral, parasacral, and paravesical sites, and these cases are often misdiagnosed as an ovarian tumor. Meador and McLarney5 reported 16 cases (10 women and 6 men) of CD of the
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International Journal of Surgical Pathology 22(7)
Figure 3. Immunohistochemical analysis. Immunohistochemical staining show (A) CD21 and (B) epidermal growth factor receptor positivity within expanded follicular dendritic cells network (40×).
pelvis, in which 9 cases were found in the retroperitoneum or pelvis, 3 in the porta hepatis, and 3 in the mesentery. One patient showed generalized lymphadenopathy in the chest with diffuse abdominal lymph nodes. Histologically, the HV type was found in 11 patients, the plasma cell type in 3 cases, and the mixed type in 2 patients. In our case, the lesion was confined to the right ovary, and lymph node enlargement and mass-like lesions were not identified. Differential diagnosis of HVCD includes follicular lymphoma (FL), mantle cell lymphoma (MCL), IgG4related sclerosing disease, and reactive follicular hyperplasia. In FL, neoplastic follicles are closely packed GCs, including centrocytes and centroblasts, present in variable proportions without a mantle zone. HV regions are rare and interfollicular regions are not as vascularized as in HVCD.6 Immunohistochemical findings show follicles and interfollicular areas containing neoplastic B-cells positive for monoclonal immunoglobulin light chain, CD10, and Bcl-2. MCL can mimic HVCD by presenting a wide mantle zone pattern of neoplastic B-cells. However, MCL lacks the prominent vascularity and clusters of plasmacytoid cells in the interfollicular regions.6 Moreover, the neoplastic B-cells show positive staining for CD5 and cyclin D1. IgG4-related sclerosing disease is characterized by dense lymphoplasmacytic cell infiltration in which the infiltrating plasma cells are IgG4-positive. In reactive follicular hyperplasia, numerous enlarged follicles of various size and shape are distributed in a pattern that causes the formation of light and dark zones. However, it is quite rare that reactive follicular hyperplasia presents with an angiofollicular pattern consisting of a whorl-like arrangement of lymphocytes with penetration by hyalinized vessels.7 Recently, EGFR expression in FDCs was reported in the follicular dendritic sarcoma and CD.8,9 In FL and
MCL, EGFR expression was absent in the FDCs. Expression of EGFR also largely lacked in the FDCs of GCs of reactive lymph node.8 The present case showed moderate positivity for EGFR by immnostaining and the reported cases of CD usually demonstrated moderate or strong EGFR expression.8,9 In conclusion, we report the first case of HVCD localized in the ovary. Clinically, computed tomography and magnetic resonance imaging have proven to be helpful in the diagnosis of pelvic tumors; however, radiographically and operatively, CD is indistinguishable from other diseases. Therefore, a surgical resection and conventional histopathological evaluation can provide an accurate characterization of the tumor. Declaration of Conflicting Interests The author(s) declared no conflict of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Keller AR, Hochhlzer L, Castleman B. Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer. 1972;29:670-683. 2. Frizzera G. Castleman’s disease and related disorders. Semin Diagn Pathol. 1988;5:346-364. 3. Ylinen K, Sarlomo-Rikala M, Laatikainen T. Pelvic Castleman disease mimicking an adnexal tumor. Obstet Gynecol. 1995;85:894-897. 4. Nakamura Y, Tokuyama O, Muso A, Kawamura N, Yasui T, Ishiko O. Asymptomatic pelvic Castleman disease in an infan-
Hatanaka et al tile woman: case report. Arch Gynecol Obstet. 2004;269:156158. 5. Meador TL, McLarney JK. CT features of Castleman disease of the abdomen and pelvis. AJR Am J Roentgenol. 2000;175:115-118. 6. Ioachim HL, Medeiros LJ, eds. Ioachim’s Lymph Node Pathology. 4th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2009. 7. Tanizawa T, Eishi Y, Kamiyama R, et al. Reactive lymphoid hyperplasia of the liver characterized by an angiofollicular
655 pattern mimicking Castleman’s disease. Pathol Int. 1996;46:782-786. 8. Sun X, Chang KC, Abruzzo LV, Lai R, Younes A, Jones D. Epidermal growth factor expression in follicular dendritic cells: a shared feature of follicular dendritic cell sarcoma and Castleman’s disease. Hum Pathol. 2003;34:835-840. 9. Lee J, Ban JY, Won KY, et al. Expression of EGFR and follicular dendritic markers in lymphoid follicles from patients with Castleman’s disease. Oncol Rep. 2008;20:851-856.
Copyright of International Journal of Surgical Pathology is the property of Sage Publications Inc. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
research-article2014
IJSXXX10.1177/1066896913517936International Journal of Surgical PathologyHatanaka et al
Case Report
The Hyaline Vascular Type of Castleman’s Disease of the Ovary: A Case Report
International Journal of Surgical Pathology 2014, Vol. 22(7) 652–655 © The Author(s) 2014 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/1066896913517936 ijs.sagepub.com
Kazuhito Hatanaka, MD1, Takako Yoshioka, MD1, Takashi Tasaki, MD1, and Akihide Tanimoto, MD1
Abstract Castleman’s disease in the pelvic cavity is rare. We present a 72-year-old woman with hyaline vascular type of Castleman’s disease of the right ovary. Right ovarian enlargement was detected in the medical examination. Computed tomography revealed a solid mass, measured 2.5 cm in size, in the right ovary. A bilateral salpingo-oophorectomy was performed. Morphologically, lymphoid follicles with regressed germinal centers (GCs) were surrounded by a broad mantle zone composed of concentric rings of small lymphocytes, and the hyalinized blood vessels with plump endothelial cells penetrated radially into GCs. Proliferation of follicular dendritic cells, which were positive for CD21 and epidermal growth factor receptor, were detected in GCs and mantle zone. No other lesions were found. To the best of our knowledge, this is the first case of hyaline vascular type of Castleman’s disease of ovarian primary. Keywords Castleman’s disease, hyaline vascular type, ovary
Introduction Castleman’s disease (CD) is histologically divided into 2 forms: hyaline vascular (HV) type, which is the predominant form that accounts for approximately 90% of cases, and plasma cell type.1 The HVCD frequently occurs in a thoracic lymph node, although other rare lesions (both nodal and extranodal) also develop in the larynx, vulva, intrapericardial, intracranial, subcutaneous, and intramuscular location, and so on.2 We present the first case of HVCD that primarily occurred in the ovary.
Case Report A right ovarian enlargement was detected in a 72-year-old woman in the health examination. Computed tomography revealed a solid mass of 2.5 cm in size located in the right ovary. No other masses, lymphadenopathy, and surrounding invasion were noted. A bilateral salpingo-oophorectomy was performed under laparoscope. Macroscopically, the cut surface of the resected right ovary showed whitish-yellow and solid tumor with a distinct border, approximately 2.5 cm in diameter (Figure 1). No hemorrhage and necrosis were identified. There were no findings of invasion to the adjacent organs and structures including paraovarian lymph nodes. Besides the right fallopian tube, the left adnexa were intact.
Figure 1. Macroscopic view of the ovarian mass. The cut surface of the right ovary was whitish-yellow and solid with a distinct border.
Microscopic examination showed that round or irregular-shaped lymphoid follicles proliferated with small 1
Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan Corresponding Author: Akihide Tanimoto, Department of Molecular and Cellular Pathology, Kagoshima University Graduate School of Medical and Dental Sciences, 8-35-1 Sakuragaoka, Kagoshima 890-8544, Japan. Email: [email protected]
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Hatanaka et al
Figure 2. Microscopic view of the ovarian mass. (A) Round or irregular-shaped lymphoid follicles with regressed germinal centers are proliferated in the ovarian tissue (arrow) (hematoxylin and eosin [H&E] stain, 4×). (B) Hyalinized blood vessels with plump endothelial cells radially penetrate into the follicles with concentric layer of mantle zone lymphocytes (“onion skin”) (H&E stain, 40×). (C) The germinal center is partially depleted of small lymphocyte and composed of follicular dendritic cells (H&E stain, 60×).
sized germinal centers (GCs), and normal ovarian tissue, including corpus albicans, was located in periphery (Figure 2A). There are some follicles with no GCs or more than one GC. Hyalinized blood vessels with plump endothelial cells penetrated radially into the follicles and a broad mantle zone, composed of concentric rings of small lymphocytes, was observed (Figure 2B). The GCs are depleted of small lymphocytes and composed of follicular dendritic cells (FDCs; Figure 2C). Hyaline deposits were also found. In the interfollicular area, small lymphoid cell infiltration, plasmacytoid dendritc cells clusters, and blood vessels proliferation lined by plump endothelial cells, but no prominent plasma cell infiltration, were identified. There was no rim of normal lymph node around the lesion. Immunohistochemically, the follicles were composed of CD20-positive B-cells. CD21 immunostaining revealed expanded FDCs network (Figure 3A).
Moreover, epidermal growth factor receptor (EGFR) expressed in FDCs (Figure 3B). The lymphocytes in the GCs were negative for Bcl-2 and cyclin D1. A few scattered plasma cells were polyclonal; immunoglobulin heavy chains (IgG, IgA, and IgM), and immunoglobulin light chains (κ and λ) were present with approximately equal distribution. Furthermore, plasma cells were negative for IgG4. T-cells positive for CD3 were predominantly found around the follicles.
Discussion Only few cases of CD in the female pelvis have been reported.3-5 They occur in the retroperitoneal space adjacent to the lateral pelvic wall as well as in presacral, parasacral, and paravesical sites, and these cases are often misdiagnosed as an ovarian tumor. Meador and McLarney5 reported 16 cases (10 women and 6 men) of CD of the
654
International Journal of Surgical Pathology 22(7)
Figure 3. Immunohistochemical analysis. Immunohistochemical staining show (A) CD21 and (B) epidermal growth factor receptor positivity within expanded follicular dendritic cells network (40×).
pelvis, in which 9 cases were found in the retroperitoneum or pelvis, 3 in the porta hepatis, and 3 in the mesentery. One patient showed generalized lymphadenopathy in the chest with diffuse abdominal lymph nodes. Histologically, the HV type was found in 11 patients, the plasma cell type in 3 cases, and the mixed type in 2 patients. In our case, the lesion was confined to the right ovary, and lymph node enlargement and mass-like lesions were not identified. Differential diagnosis of HVCD includes follicular lymphoma (FL), mantle cell lymphoma (MCL), IgG4related sclerosing disease, and reactive follicular hyperplasia. In FL, neoplastic follicles are closely packed GCs, including centrocytes and centroblasts, present in variable proportions without a mantle zone. HV regions are rare and interfollicular regions are not as vascularized as in HVCD.6 Immunohistochemical findings show follicles and interfollicular areas containing neoplastic B-cells positive for monoclonal immunoglobulin light chain, CD10, and Bcl-2. MCL can mimic HVCD by presenting a wide mantle zone pattern of neoplastic B-cells. However, MCL lacks the prominent vascularity and clusters of plasmacytoid cells in the interfollicular regions.6 Moreover, the neoplastic B-cells show positive staining for CD5 and cyclin D1. IgG4-related sclerosing disease is characterized by dense lymphoplasmacytic cell infiltration in which the infiltrating plasma cells are IgG4-positive. In reactive follicular hyperplasia, numerous enlarged follicles of various size and shape are distributed in a pattern that causes the formation of light and dark zones. However, it is quite rare that reactive follicular hyperplasia presents with an angiofollicular pattern consisting of a whorl-like arrangement of lymphocytes with penetration by hyalinized vessels.7 Recently, EGFR expression in FDCs was reported in the follicular dendritic sarcoma and CD.8,9 In FL and
MCL, EGFR expression was absent in the FDCs. Expression of EGFR also largely lacked in the FDCs of GCs of reactive lymph node.8 The present case showed moderate positivity for EGFR by immnostaining and the reported cases of CD usually demonstrated moderate or strong EGFR expression.8,9 In conclusion, we report the first case of HVCD localized in the ovary. Clinically, computed tomography and magnetic resonance imaging have proven to be helpful in the diagnosis of pelvic tumors; however, radiographically and operatively, CD is indistinguishable from other diseases. Therefore, a surgical resection and conventional histopathological evaluation can provide an accurate characterization of the tumor. Declaration of Conflicting Interests The author(s) declared no conflict of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Keller AR, Hochhlzer L, Castleman B. Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer. 1972;29:670-683. 2. Frizzera G. Castleman’s disease and related disorders. Semin Diagn Pathol. 1988;5:346-364. 3. Ylinen K, Sarlomo-Rikala M, Laatikainen T. Pelvic Castleman disease mimicking an adnexal tumor. Obstet Gynecol. 1995;85:894-897. 4. Nakamura Y, Tokuyama O, Muso A, Kawamura N, Yasui T, Ishiko O. Asymptomatic pelvic Castleman disease in an infan-
Hatanaka et al tile woman: case report. Arch Gynecol Obstet. 2004;269:156158. 5. Meador TL, McLarney JK. CT features of Castleman disease of the abdomen and pelvis. AJR Am J Roentgenol. 2000;175:115-118. 6. Ioachim HL, Medeiros LJ, eds. Ioachim’s Lymph Node Pathology. 4th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2009. 7. Tanizawa T, Eishi Y, Kamiyama R, et al. Reactive lymphoid hyperplasia of the liver characterized by an angiofollicular
655 pattern mimicking Castleman’s disease. Pathol Int. 1996;46:782-786. 8. Sun X, Chang KC, Abruzzo LV, Lai R, Younes A, Jones D. Epidermal growth factor expression in follicular dendritic cells: a shared feature of follicular dendritic cell sarcoma and Castleman’s disease. Hum Pathol. 2003;34:835-840. 9. Lee J, Ban JY, Won KY, et al. Expression of EGFR and follicular dendritic markers in lymphoid follicles from patients with Castleman’s disease. Oncol Rep. 2008;20:851-856.
Copyright of International Journal of Surgical Pathology is the property of Sage Publications Inc. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
